Histiocytoid cardiomyopathy: a cause of sudden death in infancy

Abstract

We report the case of an infant aged of 14 months deceased of sudden death. The diagnosis of histiocytoid cardiomyopathy was made on a necropsic basis. The pathologic examination showed a cardiac hypertrophy characterized by yellowish areas with irregular outlines, disseminated in the myocardium, and made of histiocyte-like cells with foamy or granular cytoplasm. These cells reacted positively with desmin and myoglobin labels, and had rare and disorganised myofibrils in electron microscopy, proving their muscular origin. The illness affects infants and usually causes severe cardiac troubles leading to death without treatment. This case is the fourteenth associated with sudden death.